Search Results (14)

Since 1999, sellar xanthogranulomas (XGs) have been recognized as a distinct pathological entity characterized by cholesterol clefts, macrophages, chronic inflammatory infiltrates with multinucleated giant cells, necrotic debris, and hemosiderin, suggesting a chronic inflammatory process with repetitive intralesional bleeding. This study aims to characterize the clinical phenomenology of the rare XG entity and to explore its correlation with other lesions. A retrospective screening was conducted of 628 sellar surgeries performed during the 2007–2024 period at a major communal hospital in Berlin and 529 surgeries between 2015 and 2024 at Ulm University. Eight XGs were analyzed. Eight XGs (0.6% of 1157 surgeries) showed mixed intra- and suprasellar localization. Visual deficits and endocrinological insufficiencies were the most common symptoms (four out of eight each). Visual recovery was favorable (three out of four complete, one out of four marked improvement), whereas endocrinological recovery was limited (one out of four). One patient experienced recurrence. Intraoperatively, seven out of eight lesions contained characteristic fluid described as ‘golden water of Gdansk.’ Postoperatively, transient arginine vasopressin deficiency occurred in four out of eight patients. The illustrative case demonstrated transformation of a Rathke’s cleft cyst, while two cases were associated with pituitary adenomas. Sellar XGs are benign, chronic inflammatory and hemorrhagic lesions with low recurrence risk. Their frequent association with other sellar pathologies supports a secondary reactive origin. Full article

Background: In the last few decades, endoscopic endonasal approaches (EEA) have revolutionized surgical access to the sellar region and anterior cranial fossa (ACF). One technique, the endoscopic endonasal transethmoidal transcribriform approach (EETTA), offers distinct advantages over traditional open transcranial approaches, such as reduced morbidity, shorter hospital stays, faster recovery, and a reduced risk of neurological deficit due to less brain tissue manipulation. Methods: We present a comprehensive step-by-step description of the EETTA surgical technique, illustrated through four representative cases of varying pathologies treated at our institution. The anatomical boundaries—including the lamina papyracea, anterior and posterior ethmoidal arteries, and frontal sinus—and the surgical corridor are detailed alongside indications, technical nuances, limitations, and operative recommendations. Results: Four cases demonstrate the versatility of EETTA across diverse pathologies: two olfactory groove meningiomas (including one WHO grade 2 and one recurrent case with invasive skull base involvement), an esthesioneuroblastoma (ENB), and a recurrent inverted papilloma requiring combined transcranial and endoscopic resection. Near-total or gross-total resection was achieved in all cases. The indications, nuances, and limitations of this approach are discussed, along with tips for successful surgery. Conclusions: The EETTA represents an important minimally invasive option for ACF tumors extending into the nasal cavity, with midline involvement limited medially by the lamina papyracea. Success requires a thorough understanding of skull base anatomy, meticulous multilayer reconstruction techniques, and appropriate patient selection, based on the tumor location and lateral extension. While cerebrospinal fluid (CSF) leak remains the primary concern, contemporary techniques have substantially reduced this complication rate. Full article

Background: The sphenoid sinus is essential for transsphenoidal surgical accesses to the sellar and parasellar regions because of its anatomic proximity to vital vascular and neurologic structures such as the internal carotid artery, optic nerve, and cavernous sinus. The high degree of morphological variability of the sphenoid sinus has a significant impact on surgical technique and the risk of intraoperative complications. Detailed knowledge of individual anatomy is therefore crucial for the safety and efficacy of transsphenoidal approaches. Objectives: This review aims to conduct a systematic analysis of the current scientific literature on anatomical variations in the sphenoid sinus and their clinical relevance in surgical interventions to the skull base. Special attention is paid to the influence of morphological features on surgical strategies to pathological processes in this area and postoperative outcomes. Materials and Methods: A systematic review of the literature was conducted according to PRISMA 2020 guidelines. The PubMed, Scopus, Web of Science, and Google Scholar databases were searched for the period March 2010 to March 2025. Keywords such as “sphenoid sinus”, “anatomical variations”, “transsphenoidal surgery” and “skull base” were used. Original studies, systematic reviews, and meta-analyses focused on the anatomy, pneumatization, and surgical significance of sphenoid sinus variations are included. Quality and relevance criteria for published material were considered in the selection of articles. Results: The most commonly identified anatomic variations included sellar and lateral pneumaticity, the presence of Onodi cells, multiple and deviated septa, and dehiscence of the posterior wall of the sphenoid sinus and prolapse into its cavity of the internal carotid artery. These variations are associated with an increased risk of intraoperative vascular injury, visual deficit, and postoperative liquorrhea. Accurate preoperative assessment by high-resolution computed axial tomography and magnetic resonance imaging, as well as the use of intraoperative neuronavigation, are critical to reduce surgical risk. Conclusions: Anatomic variations in the sphenoid sinus are an essential factor to consider when planning and performing transsphenoidal surgical accesses. An individualized approach based on detailed diagnostic imaging analysis and neuronavigation technologies contributes to a higher safety of the performed surgical interventions, a better radicality of tumor resection and more favorable postoperative outcomes. Full article

Background/Objectives: Non-functional pituitary macroadenomas (NFPMA) are uncommon pituitary lesions that do not cause hormonal hypersecretion and are most often discovered at the macroadenoma stage. Consequently, they are more challenging to diagnose, often mimicking other non-secreting sellar masses, among which hypophysitis should be carefully considered. This study aimed to differentiate between non-functioning pituitary macroadenomas (NFPMA) and hypophysitis, two distinct sellar pathologies with overlapping MRI features, by developing a diagnostic score based on clinical, biological, and radiological criteria. Methods: We conducted a prospective study, including 56 patients with NFPMA and 16 patients with hypophysitis primarily of the lymphocytic subtype. A total of 31 clinical, biological, and radiological variables were analyzed using univariate and multivariate statistical methods to identify significant predictors and to establish a diagnostic score. Results: Nine significant criteria were identified: female sex, headaches, visual disturbances, corticotropic insufficiency, pituitary volume ≤ 7 cm³, loss of the posterior pituitary bright spot, cavernous sinus invasion, optic pathway compression, and pituitary stalk thickening. The established score demonstrated significant performance in predicting the diagnosis of hypophysitis (p < 0.001; Area Under the Curve = 0.967; 95% CI = 0.926–1). The sensitivity and specificity of this score were 93.8% and 87.5%, respectively, using a threshold ≥0.5. The median score was −2 (interquartile range = [−3.5; 0.5]), with extremes ranging from −6.5 to 9. Among these, pituitary stalk thickening emerged as a key diagnostic indicator. Conclusions: This simple and effective multi-parametric score enables rapid and accurate differentiation of hypophysitis from NFPMA, helping to avoid unnecessary surgical interventions and to improve the management of pituitary insufficiencies and may be especially valuable in settings when biopsy is unavailable or risky. Full article

Background/Objectives: Granular cell tumors (GCTs) are rare neurogenic tumors with Schwann cell differentiation. Although most are benign, 1–2% exhibit malignant behavior. The imaging features of GCTs remain poorly characterized due to their rarity and anatomic variability. This study aims to elucidate the manifestations of GCTs in multimodal imaging across different anatomic locations. Methods: We retrospectively analyzed 66 histopathologically confirmed GCT cases (2011–2024), assessing their clinical presentations, pathological characteristics, and imaging findings from ultrasound (n = 31), CT (n = 14), MRI (n = 8), and endoscopy (n = 15). Two radiologists independently reviewed the imaging features (location, size, morphology, signal/density, and enhancement). Results: The cohort (mean age: 42 ± 12 years; 72.7% female) showed tendency in location towards soft tissue (48.4%), the digestive tract (30.3%), the respiratory system (7.6%), the breasts (7.6%), and the sellar region (6.1%). Six cases (9.1%) were malignant. The key imaging findings by modality were as follows: Ultrasound: Well-circumscribed hypoechoic masses in soft tissue (96.1%) and irregular margins in the breasts (80%, BI-RADS 4B) were found. MRI: The sellar GCTs exhibited T1-isointensity, variable T2-signals (with 50% showing “star-like crack signs”), and heterogeneous enhancements. The soft tissue GCTs were T1-hypointense (75%) with variable T2-signals. CT: Pulmonary/laryngeal GCTs appeared as well-defined hypodense masses with mild/moderate enhancements. Endoscopy: Submucosal/muscularis hypoechoic nodules with smooth surfaces were found. Malignant GCTs were larger (mean: 93 mm vs. 30 mm) but lacked pathognomonic imaging features. Three malignant cases demonstrated metastases. Conclusions: GCTs exhibit distinct imaging patterns based on their anatomical location. While certain features (e.g., star-like crack signs) are suggestive, imaging cannot reliably differentiate benign from malignant variants. Histopathological confirmation remains essential to diagnosis, particularly given the potential for malignant transformations (at 9.1% in our series). Multimodal imaging guides the localization and biopsy planning, but clinical–radiological–pathological correlation is crucial for the optimal management. Full article

Cystic lesions are common lesions of the sellar region with various pathological types, including pituitary apoplexy, Rathke’s cleft cyst, cystic craniopharyngioma, etc. Suggested surgical approaches are not unique when dealing with different cystic lesions. However, cystic lesions with different pathological types were hard to differentiate on MRI with the naked eye by doctors. This study aimed to distinguish different pathological types of cystic lesions in the sellar region using preoperative magnetic resonance imaging (MRI). Radiomics and deep learning approaches were used to extract features from gadolinium-enhanced MRIs of 399 patients enrolled at Peking Union Medical College Hospital over the past 15 years. Paired imaging differentiations were performed on four subtypes, including pituitary apoplexy, cystic pituitary adenoma (cysticA), Rathke’s cleft cyst, and cystic craniopharyngioma. Results showed that the model achieved an average AUC value of 0.7685. The model based on a support vector machine could distinguish cystic craniopharyngioma from Rathke’s cleft cyst with the highest AUC value of 0.8584. However, distinguishing cystic apoplexy from pituitary apoplexy was difficult and almost unclassifiable with any algorithms on any feature set, with the AUC value being only 0.6641. Finally, the proposed methods achieved an average Accuracy of 0.7532, which outperformed the traditional clinical knowledge-based method by about 8%. Therefore, in this study, we first fill the gap in the existing literature and provide a non-invasive method for accurately differentiating between these lesions, which could improve preoperative diagnosis accuracy and help to make surgery plans in clinical work. Full article

Purpose: the aim of this multicenter study is to preliminarily assess the role of the Endoscopic Endonasal Approach (EEA) in ultra-early (i.e., within 12 h) management of selected neurosurgical emergencies in terms of clinical and radiological outcomes. Methods: 26 patients affected by sellar/parasellar pathologies with rapid progression of symptoms were managed with EEA within 12 h from diagnosis in three Italian tertiary referral Centers from January 2016 to December 2019. Both clinical and radiological data have been collected preoperatively as well as post-operatively in order to perform retrospective analysis. Results: The average time from admission to the operating room was 5.5 h (±2.3). The extent of resection was gross-total in 20 (76.9%), subtotal in 6 (23.1%) patients. One patient experienced re-bleeding after a subtotal removal of a hemorrhagic lesion. Patients with a longer time from admission (>4 h) to the operatory room (OR) experienced stable impairment of the visual acuity (p = 0.033) and visual field (p = 0.029) in the post-operative setting. Conclusions: The Endoscopic Endonasal Approach represents a safe, effective technique that can be efficiently used with good results in the management of selected neurosurgical emergencies in centers with adequate experience. Full article

The sellar and parasellar region can be affected by diverse pathologies. The deep-seated location and surrounding critical neurovascular structures make treatment challenging; there is no singular, optimal approach for management. The history and development of transcranial and transsphenoidal approaches by pioneers in skull base surgery were largely aimed at treating pituitary adenomas, which are the most common lesions of the sella. This review explores the history of sellar surgery, the most commonly used approaches today, and future considerations for surgery of the sellar/parasellar region. Full article

Background and Objectives: Pituitary abscess (PA) is a rare occurrence, representing less than 1% of pituitary lesions, and is defined by the presence of an infected purulent collection within the sella turcica. Pas can be classified as either primary, when the underlying pituitary is normal prior to infection, or secondary, when there is associated a pre-existing sellar pathology (i.e., pituitary adenoma, Rathke’s cleft cysts, or craniopharyngioma), with or without a recent history of surgery. Preoperative diagnosis, owing to both non-specific symptoms and imaging features, remains challenging. Treatment options include endonasal trans-sphenoidal pus evacuation, as well as culture and tailored antibiotic therapy. Methods: A retrospective multicenter study, conducted on a prospectively built database over a 20-year period, identified a large series of 84 patients harboring primary sellar abscess. The study aimed to identify crucial clinical and imaging features in order to accelerate appropriate management. Results: The most common clinical presentation was a symptom triad consisting of various degrees of asthenia (75%), visual impairment (71%), and headache (50%). Diagnosis was achieved in 95% of cases peri- or postoperatively. Functional recovery was good for visual disturbances and headache. Pituitary function recovery remained very poor (23%), whereas the preoperative diagnosis represented a protective factor. Conclusions: In light of the high prevalence of pituitary dysfunction following the management of PAs, early diagnosis and treatment might represent a crucial issue. Currently, there are no standard investigations to establish a conclusive preoperative diagnosis; however, new, emerging imaging methods, in particular nuclear imaging modalities, represent a very promising tool, whose potential warrants further investigations. Full article

Introduction: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective case series, we describe our experience in treating pediatric skull base lesions and provide a systematic overview of the literature on the treatment and outcome of pediatric skull base lesions. Methods: We conducted a retrospective data collection of all pediatric patients (<18 years) treated for a skull base lesion at the Division of Pediatric Neurosurgery, University Children’s Hospital Basel, Switzerland, between 2015 and 2021. Descriptive statistics and a systematic review of the available literature were additionally conducted. Results: We included 17 patients with a mean age of 8.92 (±5.76) years and nine males (52.9%). The most common entity was sellar pathologies (n = 8 47.1%), with craniopharyngioma being the most common pathology (n = 4, 23.5%). Endoscopic approaches, either endonasal transsphenoidal or transventricular, were used in nine (52.9%) cases. Six patients (35.3%) suffered from transient postoperative complications, while in none of the patients these were permanent. Of the nine (52.9%) patients with preoperative deficits, two (11.8%) showed complete recovery and one (5.9%) partial recovery after surgery. After screening 363 articles, we included 16 studies with a total of 807 patients for the systematic review. The most common pathology reported in the literature confirmed our finding of craniopharyngioma (n = 142, 18.0%). The mean PFS amongst all the studies included was 37.73 (95% CI [36.2, 39.2]) months, and the overall weighted complication rate was 40% (95% CI [0.28 to 0.53] with a permanent complication rate of 15% (95% CI [0.08 to 0.27]. Only one study reported an overall survival of their cohort of 68% at five years. Conclusion: This study highlights the rarity and heterogeneity of skull base lesions in the pediatric population. While these pathologies are often benign, achieving GTR is challenging due to the deep localization of the lesions and eloquent adjacent structures, leading to high complication rates. Therefore, skull base lesions in children require an experienced multidisciplinary team to provide optimal care. Full article

Objective: The Ki-67 index is an indicator of the active proliferation and aggressive behavior of pituitary adenomas (PAs). Appropriate pre- and intra-operatives of the Ki-67 index can help surgeons develop better and more personalized treatment strategies for patients with PAs. This study aimed to investigate the influence factors for predicting the Ki-67 index in PAs. Methods: Data of 178 patients with PAs confirmed by pathology were retrospectively analyzed. According to the Ki-67 index, the patients were divided into the Ki-67 < 3% and Ki-67 ≥ 3% cohorts. Patient data, including age, sex, postoperative immunohistochemical pituitary hormone positive index, Knosp grade, tumor breaking through the sellar floor, rich blood supply to the tumor, tumor located inside the sella, erosion of the dorsum sellae bone, and pituitary-specific transcription factor, were collected. A univariate logistic analysis was used to evaluate the influence factors for a high Ki-67 index. Multiple regression and receiver operating characteristic (ROC) curve were used to analyze the factors with p < 0.05. The mutant status of Ki-67 index was predicted by nomogram. Results: Multivariate regression analysis showed that rich blood supply to the tumor and erosion of the dorsum sellae bone were independent risk factors for the Ki-67 proliferation index. The ROC curves demonstrated that age, rich blood supply to the tumor, and erosion of the dorsum sellae bone can predict the occurrence of a high Ki-67 index. Together, the three risk factors provide a stronger ability to predict the Ki-67 index. The nomogram was developed and validated. Conclusion: Age, rich blood supply to the tumor, and erosion of the dorsum sellae bone are influencing factors for predicting the Ki-67 index. Suitable nomogram prediction models were developed and validated, and there is potential for personalized treatment for PA patients. Full article

An excess of growth hormone (GH) results in accelerated growth and in childhood, the clinical manifestation is gigantism. When GH excess has its onset after epiphyseal fusion at puberty, the overgrowth of soft tissue and bone results in acromegaly. Persistent GH excess in gigantism also causes acromegalic features that become evident in the adult years. The causes of GH excess are primarily lesions in the pituitary, which is the main source of GH. In this review, we provide an update on the clinical, radiological and pathologic features of the various types of pituitary neuroendocrine tumors (PitNETs) that produce GH. These tumors are all derived from PIT1-lineage cells. Those composed of somatotrophs may be densely granulated, resembling normal somatotrophs, or sparsely granulated with unusual fibrous bodies. Those composed of mammosomatotrophs also produce prolactin; rare plurihormonal tumors composed of cells that resemble mammosomatotrophs also produce TSH. Some PitNETs are composed of immature PIT1-lineage cells that do not resemble differentiated somatotrophs, mammosomatotrophs, lactotroph or thyrotrophs; these tumors may cause GH excess. An unusual oncocytic PIT1-lineage tumor known as the acidophil stem cell tumor is predominantly a lactotroph tumor but may express GH. Immature PIT1-lineage cells that express variable amounts of hormones alone or in combination can sometimes cause GH excess. Unusual tumors that do not follow normal lineage differentiation may also secrete GH. Exceptional examples of acromegaly/gigantism are caused by sellar tumors composed of hypothalamic GHRH-producing neurons, alone or associated with a sparsely granulated somatotroph tumor. Each of these various tumors has distinct clinical, biochemical and radiological features. Data from careful studies based on morphologic subtyping indicate that morphologic classification has both prognostic and predictive value. Full article

In the present study, the results of brain magnetic resonance imaging (MRI) in girls with central precocious puberty (CPP) were compared those in with girls evaluated for headaches. A total of 295 girls with CPP who underwent sellar MRI were enrolled. A total of 205 age-matched girls with chronic or recurrent headaches without neurological abnormality who had brain MRI were included as controls. The positive MRI findings were categorized as incidental non-hypothalamic–pituitary (H–P), incidental H–P, or pathological. Positive MRI findings were observed in 39 girls (13.2%) with CPP; 8 (2.7%) were classified as incidental non-H–P lesions, 30 (10.2%) as incidental H–P lesions, and 1 (0.3%) as a pathological lesion (tuber cinereum hamartoma). The prevalence of positive MRI findings in girls with CPP did not differ from girls with headaches (13.2% vs. 12.2%, p = 0.74). The prevalence of incidental H–P lesions in girls with CPP <6 years of age, 6–6.9 years of age, and 7–7.9 years of age was 21.2%, 13.5%, and 9.6%, respectively (p = 0.21). Known pathological lesions were detected in only one (3.0%) girl with CPP aged <6 years and in no girls with CPP aged 6–7.9 years. Microadenomas were detected in no girls with CPP aged <6 years and in 5 (1.9%) girls with CPP aged of 6–7.9 years. Our findings call into question the routine use of brain MRI in girls with CPP, especially in girls 6 years or older. Current guidelines recommend a follow-up MRI in cases of microadenoma, but few data exist to support this recommendation for children. Full article

Hyperprolactinaemia is one of the most common problems in clinical endocrinology. It relates with various aetiologies (physiological, pharmacological, pathological), the clarification of which requires careful history taking and clinical assessment. Analytical issues (presence of macroprolactin or of the hook effect) need to be taken into account when interpreting the prolactin values. Medications and sellar/parasellar masses (prolactin secreting or acting through “stalk effect”) are the most common causes of pathological hyperprolactinaemia. Hypogonadism and galactorrhoea are well-recognized manifestations of prolactin excess, although its implications on bone health, metabolism and immune system are also expanding. Treatment mainly aims at restoration and maintenance of normal gonadal function/fertility, and prevention of osteoporosis; further specific management strategies depend on the underlying cause. In this review, we provide an update on the diagnostic and management approaches for the patient with hyperprolactinaemia and on the current data looking at the impact of high prolactin on metabolism, cardiovascular and immune systems. Full article